Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Flashcards
How does a lymphoma present clinically?
(regardless of type!)
Painless lymphadenopathy
* commonly neck, axilla, groin
* if intracavity lymph node, then cannot be palpated but obstructive symptoms can develop!
* pain after alcohol = hodgkin’s (does not occur in NHL)
Organ infiltration
* Ocular lymphoma
* skin nodules - mucosis fungoides
Recurrent infections
* lymphocytes are ineffective therefore poor immune reaction
FLAWS
* B symptoms in high grade aggressive lymphomas due to increased metabolic state.
What obstructive symptoms can arise from a lymphoma causing an enlarged intra-cavity lymph node
Ureter obstruction –> renal failure
Bile duct obstruction –> obstructive jaundice
IVC / SVC —> oedema
Tracheal obstruction –> respiratory distress
What are the important investigations to carry out when diagnosing a lymphoma
Histology: biopsy + immunophenotype + molecular tools
- Crucial for dx of type of lymphoma + prognosis
Anatomical Staging: PET-CT + Bone marrow biopsy (if bone marrow could be affected) + Lumbar puncture (if CNS involvement likely)
Bloods:
* LDH - marker of cell turnover
* Albumin - important for liver function
* HIV serology - HIV can predispose to NHL (HTLV1 serology may also be important)
* Hepatitis B serology - Lymphoma treatment may deplete B cells resulting in fulminant liver failure due to reactivation of hepatitis B in chronic carriers
Demographics of a typical patient with Hodgkin’s Lymphoma
Bimodal age distribution
- 20-29 (commonly female = nodular sclerosing HL)
- >60 (commonly male)
Overall commonly male
Describe the typical presentation of Hodgkin’s lymphoma.
Painless lymphadenopathy
* With contigous spread.
* Pain on alcohol
* nodes tend to mediastinal/cervical!
Compression symptoms
B symptoms
Outline the staging system used for Hodgkin’s Lymphoma
Done using PET-CT scan - allows to see highly active lymph nodes (note that kidneys and bladder will be lit up due to excretion not!)
Ann Arbor Staging
Stage 1 - one LN region (Ln region can include spleen!)
Stage 2 - two or more LN regions same side of diaphragm
Stage 3 - two or more LN regions both sides of diaphragm
Stage 4 - involvement of extra nodal sites (Liver, BM)
+
A: no constitutional symptoms
B: constitutional symptoms
Outline the management for Hodgkin’s Lymphoma
- Combination therapy (ABVD) 2-6 cycles. After 2nd cycle a PET-CT is done to check treatment efficacy (depending on it do more or less)
- Radiotherapy: may be used alongside chemo in bulky areas but VERY HIGH RISK OF BREAST CANCER!
For relapsed patients: second line chemo agents or stem cell transplant.
What are the two most common types of non-Hodgkin lymphoma?
Diffuse large B cell lymphoma (DLBCL) (30-40% of all NHL)
Follicular lymphoma (35% of all NHL)
List some types of non-Hodgkin lymphoma that are:
- Very agressive
- Aggresive
- Indolent
-
Very agressive
- Burkitt’s lymphoma
- T or B cell lymphoblastic lymphoma/leukaemia
-
Aggressive
- Diffuse large B cell lymphoma
- Mantle cell lymphoma
-
Indolent
- Follicular lymphoma
- Small lymphocytic lymphoma (CLL)
- MALToma
What is the correlation between how aggressive a lymphoma is and how curable it is?
The more aggressive it is, the more curable
Indolent lymphoma can enter remission but is more likely to recur
Which factors are taken into account by the international prognostic index (IPI) for lymphoma?
- Age >60
- High LDH
- Performance status 2-4
- Stage III or IV
- More than one extranodal site
Outline the staging method used for NHL
Ann Arbor staging - the same as Hodgkin’s lymphoma
Broadly speaking, what are the treatment options to non-Hodgkin lymphomas?
- Monitor only (in indolent lymphoma)
- Urgent chemotherapy
- Non-chemotherapy treatment (e.g. Antibiotic for H. Pylori)
Which chemotherapy treatment is usually used for diffuse large B cell lymphoma?
- R-CHOP - 6-8 cycles
- Rituximab + CHOP (chemo agents)
Achieves a 50% cure rate. Failure to achieve cure rate = Auto-SCT or CAR-T (T-cell therapy)
What treatment option may be considered for patients with diffuse large B cell lymphoma who relapse?
Autologous stem cell transplantation
What is the usual first-line treatment approach to follicular lymphoma? Outline the indications to escalate treatment
Watch and wait
Only treat it clinically indicated (e.g. compression symptoms, massive nodes, recurrent infection)
Which chemotherapy regimen may be used in the treatment of follicular lymphoma?
- R-CVP
- Rituximab
- Cyclophosphamide
- Vincristine
- Prednisolone
Which lymphoid tissue tends to be affected by marginal zone lymphoma?
Extranodal lymphoid tissue (e.g. MALT)
What is the cause of marginal zone lymphoma?
- H. pylori infection - gastric MALToma
- Sjogren’s syndrome - parotid lymphoma
- Hashimoto’s thyroiditis - thyroid lymphoma
- Psittaci infection - lacrimal gland
Where is marginal zone lymphoma most commonly seen and how does it tend to present?
- Usually in the stomach
- Presenting with dyspepsia or epigastric pain
- Usually Stage 1{E} (E=extranodal)
- B symptoms are uncommon
Outline the process of Gastric MALT lymphomagenesis.
- Lymphocytes will respond to H. pylori infection and proliferate
- At some point, they will over-proliferate and develop cancer-like features but they will still be dependent on antigenic stimulation by H. pylori
- At this point, treating H. pylori will treat the lymphoma
How might gastrict MALToma stage I-II disease be treated?
- Triple therapy to eradicate H. pylori (2 antibiotics + 1 PPI)
- Repeat breath test at 2 months
- Repeat endoscopy every 6 months for 1-2 years then annually
NOTE: failure may require chemotherapy
What are the main features of enteropathy-associated T cell lymphoma?
- Mature T cells
- Involves small intestines
- Aggressive
- Caused by chronic antigenic stimulation by gliadin/gluten
Describe the typical presenting features of enteropathy-associated T cell lymphoma.
- Abdominal pain/ obstruction/ bleeding/ perforation
- Malabsorption
- Systemic symptoms
